Unmet health care needs in immune thrombotic thrombocytopenic purpura survivors

Immune thrombotic thrombocytopenic purpura (iTTP) is a rare thrombotic microangioathy episodic life-threatening thrombotic microangiopathy. Survivors of iTTP have high rates of chronic morbidities in remission such as depression, cognitive impairment, stroke, and increased cardiovascular mortality. Careful multidisciplinary management during clinical remission is needed to monitor for relapse and manage comorbidities, but this may not be universally available.

To identify gaps in the outpatient care of iTTP survivors.

We conducted a cross-sectional survey-based study. The questionnaire included demographic information, disease-specific information, treatment-specific information, and the Healthcare Access and Utilization Survey developed by the National Institutes of Health All of Us Research Program.

A total of 281 participants responded to the survey. Median age was 47 years (IQR, 34-60 years). Most respondents were women (255/278, 91.7%), White (206/281, 73.7%), and highly educated with 215 of 263 (81.7%) reporting some college or further education. A quarter of respondents, 67 of 247 (27.1%), had not seen a hematologist in the past year, and 48 of 247 (19.4%) had not seen a primary care provider. Additionally, 45 of 279 (16.1%) reported not having an iTTP doctor. Laboratory monitoring was performed every 6 months or less frequently in 38% of respondents. Treatment for relapse prevention was not discussed with 54.8%. While 82.7% self-reported mental health disturbance after iTTP diagnosis, 30.4% had been referred to a mental health professional.

In this United States-based cohort, despite high rates of medical and psychiatric comorbidities, only a minority reported high-quality relapse-prevention care. There were high rates of mental health disturbances after iTTP diagnosis but limited access to mental health care.